Cystic lymphangiomatosis of the spleen is an extremely rare pathologic entity. We report the radiologic, sonographic, and scintigraphic findings in two cases of splenic cystic lymphangiomatosis. In both patients echograms demonstrated multiloculated cystic lesions in the intra-abdominal masses, which were identified on radiocolloid scans as abnormal spleens. The gross and microscopic findings of the excised spleens were characteristic of cystic lymphangiomatosis. The findings on echograms and scintigrams correlated well with the abnormal splenic morphology. The clinical presentation, the findings on diagnostic imaging, and the gross and microscopic appearances of the pathologic spleens in our patients were remarkably similar. When a massively enlarged spleen with a multicystic appearance is encountered during a diagnostic workup, awareness of the scintiscan and sonographic findings might aid in the noninvasive preoperative recognition of cystic lymphangiomatosis.

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